Abstract

The aim of the study was to analyze the efficacy and toxicity of the therapy with pulse dose of cyclophosphamide in patients with Wegener's granulomatosis. The diagnosis was established in three patients (2 male, 1 female, average age 58.3 years) upon the clinical picture, laboratory-immunologic and histopathologic findings. The initial therapy was conducted by pulse doses of cyclophosphamide (0.6 and 0.8 g/kg), glucocorticoids (0.5 g), repeated plasmapheresis and pulse doses of immunoglobulin (0.4 g/kg). All patients achieved remission with complete recovery of renal function in one patient. The therapy with pulse doses of cyclophosphamide (time-period between pulses from 1 to 3 months), together with glucocorticoids taken orally for 24 months, was continued for the following 18 months. Stable remission (4-15 months) was maintained in all the patients after the immunosuppressive therapy was over. The treatment of Wegener's granulomatosis by cyclophosphamide and glucocorticoid pulse doses with the application of plasmaphereses and immunoglobulin pulse doses in the initial therapy lead to the remission of progressive types of long duration.